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Article

Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.

Neuron - 7 Feb 2008

Pennuto Maria, Tinelli Elisa, Malaguti Mariachiara, Del Carro Ubaldo, D'Antonio Maurizio, Ron David, Quattrini Angelo, Feltri M Laura, Wrabetz Lawrence

Abstract excerpt

Deletion of serine 63 from P0 glycoprotein (P0S63del) causes Charcot-Marie-Tooth 1B neuropathy in humans, and P0S63del produces a similar demyelinating neuropathy in transgenic mice. P0S63del is retained in the endoplasmic reticulum and fails to be incorporated into myelin. Here we report that P0S63del is misfolded and Schwann cells mount a consequential canonical unfolded protein response (UPR), including...

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Identifiers

PMID: 18255032
DOI: 10.1016/j.neuron.2007.12.021
PMCID: PMC2267889

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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